Abstract

A 16-year-old boy with dyskeratosis congenita (DC), a very rare polymorphous inherited telomeropathy, was referred for bilateral large peripheral temporal areas of retinal nonperfusion (Fig A). Absence of associated retinal neovascularization was confirmed by fluorescein angiography (FA) (Fig B). Although peripheral retinal ischemia is known in DC, unexpected numerous reddish, rounded, preretinal microvascular tufts were also observed in the inferonasal macula. They were distinct on the near-infrared image (Fig C) and localized above the retinal surface on OCT B-scans (Fig D).

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